Childhood idiopathic thrombocytopenic purpura in Egypt and the neighboring Arab countries: a regional form with three different patterns of clinical expression.

Abstract

Childhood idiopathic thrombocytopenic purpura in the western world is essentially an acute self-limited disorder. In contrast, the clinical expression of the disease in Arab countries, as revealed by a study of 160 patients from Egypt, Saudi Arabia, Oatar and North Sudan, is heterogenous forming a spectrum that includes three distinct clinical forms: (a) the acute self-limited form, (b) the intermediate form, and (c) the chronic adulthood-like form. The relative proportions of these forms were 40. 15 and 45%, respectively. The chronic form shows limited response to steroids, and runs a platelet count less than 100,000 microliters for more than 1 year, with a tendency for later spontaneous elevation in platelet counts during the first few years of a long follow-up. The intermediate form shows a transient steroid-induced complete remission giving place to widely fluctuating platelet counts above and below 100,000 microliters once the steroid dosage is reduced to maintenance levels. Platelet counts in excess of 100,000 microliters were achieved in this group by extending steroid maintenance therapy fo 6--9 months. In spite of a tendency to chronicity and partial resistance to steroids i the intermediate and chronic forms, the overall response to steroids was enough both to reduce the number of cases requiring splenectomy to 15%, and to prevent the development of major complications in all the children included in the study.